Search results for "muscle protein"

Endocytosis in skeletal muscle fibers.

1999

Defining the organization of endocytic pathway in multinucleated skeletal myofibers is crucial to understand the routing of membrane proteins, such as receptors and glucose transporters, through this system. Here we analyzed the organization of the endocytic trafficking pathways in isolated rat myofibers. We found that sarcolemmal-coated pits and transferrin receptors were concentrated in the I band areas. Fluid phase markers were taken up into vesicles in the same areas along the whole length of the fibers and were then delivered into structures around and between the nuclei. These markers also accumulated beneath the neuromuscular and myotendinous junctions. The recycling compartment, lab…

Blocking of myostatin and activins increase muscle protein synthesis and mTORC1 signaling but decreases capillary density

2012

Protein aggregate myopathies.

2006

Protein aggregate myopathies (PAMs) based on the morphologic phenomenon of aggregation of proteins within muscle fibers may occur in children (selenoproteinopathies, actinopathies, and myosinopathies) or adults (certain myofibrillar myopathies and myosinopathies). They may be mutation related, which includes virtually all childhood forms but certain other forms as well, or sporadic, which are largely seen in adults. Their classification as myofibrillar or desmin-related myopathies, actinopathies, or myosinopathies is based on the identification of respective mutant proteins, most of them components of the sarcomeres. Recognition of PAM requires muscle biopsy and an extensive immunohistochem…

Congenital myopathies at their molecular dawning

2003

The introduction and application of molecular techniques have commenced to influence and alter the nosology of congenital myopathies. Long-known entities such as nemaline myopathies, core diseases, and desmin-related myopathies have now been found to be caused by unequivocal mutations. Several of these mutations and their genes have been identified by analyzing aggregates of proteins within muscle fibers as a morphological hallmark as in desminopathy and actinopathy, the latter a subtype among the nemaline myopathies. Immunohistochemistry has played a crucial role in recognizing this new group of protein aggregate myopathies within the spectrum of congenital myopathies. It is to be expected…

Nature of autoantigens and autoantibodies in autoimmune hepatitis

1990

Autoimmune chronic active hepatitis (AI-CAH) is characterized by young age at onset, predominance of females, hypergammaglobulinemia, response to immunosuppressive treatment and characteristic circulating autoantibodies. This clinical syndrome was first described by Waldenstr6m in 1950 [47]. Later the association of autoimmune hepatitis with antinuclear antibodies (ANA) lead to the term "lupoid hepatitis" [19]. Additional autoantibodies have been described [21]. At least three subgroups of AI-CAH can be distinguished serologically and clinically [28]. As diagnostic tools, autoantibodies help to further differentiate the heterogeneous group of hepatitis B virus (HBV) surface antigen (HBsAg)-…

Surplus protein myopathies.

2001

Abstract Certain muscular dystrophies are marked by absence or reduction of mutant proteins, foremost dystrophinopathies and sarcoglycanopathies. Conversely, other sporadic and familial neuromuscular conditions are marked by a surplus of proteins present in a granular or filamentous form, such as desmin-related myopathies, actinopathy and, perhaps, hyaline body myopathy. This emerging group of congenital myopathies is clinically, immunohistochemically, and genetically diverse. Clinically, early- and late-onset diseases with variable courses are described. Immunohistochemically, mutant gene-related and other proteins have been identified by immunohistochemistry. Mutations in the desmin and α…

Desmin-related neuromuscular disorders

1995

Desmin, the intermediate filament protein of skeletal muscle fibers, cardiac myocytes, and certain smooth muscle cells, is a member of the cytoskeleton linking Z-bands with the plasmalemma and the nucleus. The pathology of desmin in human neuromuscular disorders is always marked by increased amounts, diffusely or focally. Desmin is highly expressed in immature muscle fibers, both during fetal life and regeneration as well as in certain congenital myopathies, together with vimentin. Desmin is also enriched in neonatal myotonic dystrophy and small fibers in infantile spinal muscular atrophy. Focal accretion of desmin may be twofold, in conjunction with certain inclusion bodies, cytoplasmic an…

Alteration of poly (ADP-Rib) synthesis during progesterone- caused gene expression in oviducts of quails.

1976

Summary The biological model of the selective induction of RNA synthesis in oviducts of estrogen stimulated immature quails by progesterone has been used to clarify whether poly (ADP-Rib) is involved in DNA transcription. The chromatin-bound as well as the soluble poly (ADP-Rib) polymerase has been isolated from oviducts and the optimal reaction conditions have been determined. The activities, as measured by the incorporation rates of NAD + into poly (ADP-Rib), of both, chromatin-bound « endogenouspolymerase (in the absence of « exogenousDNA and histones) and soluble enzyme (native DNA - lysine-rich histone ratio: 4.3) from progesterone treated quail oviducts, have been determined to be onl…

Bipartite regulation of different components of the MHC class I antigen-processing machinery during dendritic cell maturation

2001

Dendritic cells (DC) are professional antigen-presenting cells (APC) which proceed from immature to a mature stage during their final differentiation. Immature DC are highly effective in terms of antigen uptake and processing, whereas mature DC become potent immunostimulatory cells. Until now, the expression profiles of the major components of the MHC class I antigen-processing machinery (APM) during DC development have not been well characterized. In this study, the mRNA and protein expression levels of the IFN-gamma inducible proteasome subunits, of the proteasome activators PA28, and of key components required for peptide transport and MHC class I-peptide complex assembly have been evalu…

Protein Biomarkers of Bovine Defective Meats at a Glance: Gel-Free Hybrid Quadrupole-Orbitrap Analysis for Rapid Screening

2021

An understanding of biological mechanisms that could be involved in the stress response of animal cattle prior to slaughter is critical to create effective strategies aiming at the production of high-quality meat. The sarcoplasmic proteome of directly extracted samples from normal and high ultimate pH (pHu) meat groups was studied through a straightforward gel-free strategy supported by liquid chromatography hybrid quadrupole-Orbitrap high-resolution mass spectrometry (LC-HRMS) analysis. A stepped proteomic pipeline combining rapid biomarker hunting supported by qualitative protein Mascot scores followed by targeted label-free peptide quantification revealed 26 descriptors that characterize…